{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,9,27]],"date-time":"2025-09-27T21:52:15Z","timestamp":1759009935695,"version":"3.40.4"},"reference-count":27,"publisher":"Springer Science and Business Media LLC","issue":"3","license":[{"start":{"date-parts":[[2025,2,7]],"date-time":"2025-02-07T00:00:00Z","timestamp":1738886400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/deed.de"},{"start":{"date-parts":[[2025,2,7]],"date-time":"2025-02-07T00:00:00Z","timestamp":1738886400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/deed.de"}],"funder":[{"name":"Universit\u00e4tsklinikum Leipzig"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Pathologie"],"published-print":{"date-parts":[[2025,5]]},"abstract":"<jats:title>Zusammenfassung<\/jats:title>\n          <jats:sec>\n            <jats:title>Hintergrund<\/jats:title>\n            <jats:p>Die Lymphangioleiomyomatose (LAM) ist eine seltene progrediente low-grade Neoplasie, welche \u00fcberwiegend bei jungen Frauen auftritt. Die Erkrankung ist prim\u00e4r f\u00fcr ihre pulmonale Manifestation mit zystischer Parenchymdestruktion bekannt, jedoch ist auch eine extrapulmonale Beteiligung m\u00f6glich. LAM ist assoziiert mit Mutationen in den Tuber\u00f6se-Sklerose-Komplex(TSC)-Genen <jats:italic>TSC1<\/jats:italic> und <jats:italic>TSC2<\/jats:italic> und kann sporadisch oder im Kontext der heredit\u00e4ren Erkrankung TSC auftreten.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Ziel<\/jats:title>\n            <jats:p>Sensibilisierung f\u00fcr extrapulmonale LAM-Befunde im Retroperitoneum und Beckenraum.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Methodik<\/jats:title>\n            <jats:p>Es wurde eine Datenbankrecherche zu LAM-Befunden in gyn\u00e4kologischen Operationspr\u00e4paraten durchgef\u00fchrt. HE-Schnittpr\u00e4parate wurden reevaluiert und immunhistochemische Pr\u00e4parate begutachtet. Klinische Daten wurden hinsichtlich des Vorliegens einer pulmonalen LAM oder TSC ausgewertet.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Ergebnisse<\/jats:title>\n            <jats:p>Insgesamt wurden 13\u00a0F\u00e4lle identifiziert. Das Alter der Patientinnen reichte von 32\u00a0bis 77\u00a0Jahren, wobei 8\/13\u00a0Frauen \u2264\u202f55\u00a0Jahre alt waren, 2\u00a0Patientinnen wiesen eine Anamnese von pulmonaler LAM und TSC auf. Die meisten Frauen wurden aufgrund gyn\u00e4kologischer Malignome operiert. Die histologische Begutachtung zeigte in 10\/13\u00a0F\u00e4llen LAM-L\u00e4sionen in 1\u00a0bis 9\u00a0Lymphknoten mit einer Gr\u00f6\u00dfe von 0,5\u201312,0\u202fmm, 3\/13\u00a0F\u00e4lle wiesen eine extranodale LAM-Beteiligung im Retroperitoneum, Myometrium und Ovarhilus auf. Die L\u00e4sionen exprimierten \u00fcberwiegend HMB45, Desmin und SMA.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Diskussion<\/jats:title>\n            <jats:p>LAM ist eine seltene Erkrankung mit vorrangiger Lungenbeteiligung, jedoch ist auch eine extrapulmonale Manifestation m\u00f6glich. Es ist entscheidend, LAM-Foci zu erkennen und zu berichten, um einen ersten Hinweis f\u00fcr eine bis dato nichtdiagnostizierte pulmonale LAM und\/oder eine TSC zu geben.<\/jats:p>\n          <\/jats:sec>","DOI":"10.1007\/s00292-025-01414-0","type":"journal-article","created":{"date-parts":[[2025,2,7]],"date-time":"2025-02-07T15:44:21Z","timestamp":1738943061000},"page":"179-184","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Zufallsbefund von Lymphangioleiomyomatose in gyn\u00e4kologischen Operationspr\u00e4paraten\u00a0\u2013 eine Fallserie","Incidental diagnosis of lymphangioleiomyomatosis in gynecological surgery\u2014a case series"],"prefix":"10.1007","volume":"46","author":[{"given":"Julia","family":"M\u00fcller","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Blake","family":"Gilks","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jessica","family":"McAlpine","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Grit Gesine Ruth","family":"Hiller","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anne Kathrin","family":"H\u00f6hn","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lars-Christian","family":"Horn","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2025,2,7]]},"reference":[{"issue":"10","key":"1414_CR1","doi-asserted-by":"publisher","first-page":"1370","DOI":"10.1097\/PAS.0000000000001119","volume":"42","author":"JA Bennett","year":"2018","unstructured":"Bennett\u00a0JA, Braga\u00a0AC, Pinto\u00a0A et\u00a0al (2018) Uterine PEComas: a\u00a0morphologic, immunohistochemical, and molecular analysis of 32 tumors. Am J Surg Pathol 42(10):1370\u20131383. https:\/\/doi.org\/10.1097\/PAS.0000000000001119","journal-title":"Am J Surg Pathol"},{"issue":"9869","key":"1414_CR2","doi-asserted-by":"publisher","first-page":"817","DOI":"10.1016\/S0140-6736(12)61767-X","volume":"381","author":"JJ Bissler","year":"2013","unstructured":"Bissler\u00a0JJ, Kingswood\u00a0JC, Radzikowska\u00a0E et\u00a0al (2013) Everolimus for angiomyolipoma associated with tuberous sclerosis complex or sporadic lymphangioleiomyomatosis (EXIST-2): a\u00a0multicentre, randomised, double-blind, placebo-controlled trial. Lancet 381(9869):817\u2013824. https:\/\/doi.org\/10.1016\/S0140-6736(12)61767-X","journal-title":"Lancet"},{"issue":"3","key":"1414_CR3","doi-asserted-by":"publisher","first-page":"104173","DOI":"10.1016\/j.lpm.2023.104173","volume":"52","author":"D Elia","year":"2023","unstructured":"Elia\u00a0D, Cassandro\u00a0R, Caminati\u00a0A, Luisi\u00a0F, Harari\u00a0S (2023) Lymphangioleiomyomatosis. Presse Med 52(3):104173. https:\/\/doi.org\/10.1016\/j.lpm.2023.104173","journal-title":"Presse Med"},{"key":"1414_CR4","doi-asserted-by":"publisher","DOI":"10.1530\/ERC-23-0102","author":"E Gibbons","year":"2023","unstructured":"Gibbons\u00a0E, Minor\u00a0BMN, Hammes\u00a0SR (2023) Lymphangioleiomyomatosis: where endocrinology, immunology and tumor biology meet. Endocr Relat Cancer. https:\/\/doi.org\/10.1530\/ERC-23-0102","journal-title":"Endocr Relat Cancer"},{"issue":"2","key":"1414_CR5","doi-asserted-by":"publisher","first-page":"288","DOI":"10.1016\/j.chest.2018.06.016","volume":"155","author":"N Gupta","year":"2019","unstructured":"Gupta\u00a0N, Lee\u00a0H\u2011S, Ryu\u00a0JH et\u00a0al (2019) The NHLBI LAM registry: prognostic physiologic and radiologic biomarkers emerge from a\u00a015-year prospective longitudinal analysis. Chest 155(2):288\u2013296. https:\/\/doi.org\/10.1016\/j.chest.2018.06.016","journal-title":"Chest"},{"issue":"11","key":"1414_CR6","doi-asserted-by":"publisher","first-page":"971","DOI":"10.1093\/qjmed\/hcr116","volume":"104","author":"EC Harknett","year":"2011","unstructured":"Harknett\u00a0EC, Chang\u00a0WYC, Byrnes\u00a0S et\u00a0al (2011) Use of variability in national and regional data to estimate the prevalence of lymphangioleiomyomatosis. QJM 104(11):971\u2013979. https:\/\/doi.org\/10.1093\/qjmed\/hcr116","journal-title":"QJM"},{"issue":"12","key":"1414_CR7","doi-asserted-by":"publisher","first-page":"1776","DOI":"10.1097\/PAS.0b013e318235edbd","volume":"35","author":"T Hayashi","year":"2011","unstructured":"Hayashi\u00a0T, Kumasaka\u00a0T, Mitani\u00a0K et\u00a0al (2011) Prevalence of uterine and adnexal involvement in pulmonary lymphangioleiomyomatosis: a\u00a0clinicopathologic study of 10\u00a0patients. Am J Surg Pathol 35(12):1776\u20131785. https:\/\/doi.org\/10.1097\/PAS.0b013e318235edbd","journal-title":"Am J Surg Pathol"},{"issue":"5","key":"1414_CR8","doi-asserted-by":"publisher","first-page":"1056","DOI":"10.1183\/09031936.06.00113303","volume":"27","author":"SR Johnson","year":"2006","unstructured":"Johnson\u00a0SR (2006) Lymphangioleiomyomatosis. Eur Respir\u00a0J 27(5):1056\u20131065. https:\/\/doi.org\/10.1183\/09031936.06.00113303","journal-title":"Eur Respir J"},{"issue":"1","key":"1414_CR9","doi-asserted-by":"publisher","first-page":"14","DOI":"10.1183\/09031936.00076209","volume":"35","author":"SR Johnson","year":"2010","unstructured":"Johnson\u00a0SR, Cordier\u00a0JF, Lazor\u00a0R et\u00a0al (2010) European respiratory society guidelines for the diagnosis and management of lymphangioleiomyomatosis. Eur Respir\u00a0J 35(1):14\u201326. https:\/\/doi.org\/10.1183\/09031936.00076209","journal-title":"Eur Respir J"},{"key":"1414_CR10","volume-title":"Seltene Lungenerkrankungen","year":"2022","unstructured":"Kreuter\u00a0M, Costabel\u00a0U, Herth\u00a0F, Kirsten\u00a0D (Hrsg) (2022) Seltene Lungenerkrankungen, 2.\u00a0Aufl. Springer, Berlin, Heidelberg","edition":"2"},{"issue":"2","key":"1414_CR11","doi-asserted-by":"publisher","first-page":"C320","DOI":"10.1152\/ajpcell.00202.2022","volume":"324","author":"N Kundu","year":"2023","unstructured":"Kundu\u00a0N, Holz\u00a0MK (2023) Lymphangioleiomyomatosis: a\u00a0metastatic lung disease. Am J Physiol Cell Physiol 324(2):C320\u2013C326. https:\/\/doi.org\/10.1152\/ajpcell.00202.2022","journal-title":"Am J Physiol Cell Physiol"},{"key":"1414_CR12","doi-asserted-by":"publisher","first-page":"e00650","DOI":"10.1016\/j.crwh.2024.e00650","volume":"43","author":"EM Lee","year":"2024","unstructured":"Lee\u00a0EM, Kim\u00a0JH, Jo\u00a0U, Cho\u00a0YJ (2024) Uterine lymphangioleiomyomatosis in a\u00a0premenopausal woman with tuberous sclerosis: a\u00a0case report. Case Rep Womens Health 43:e650. https:\/\/doi.org\/10.1016\/j.crwh.2024.e00650","journal-title":"Case Rep Womens Health"},{"issue":"10","key":"1414_CR13","doi-asserted-by":"publisher","first-page":"1242","DOI":"10.1053\/hupa.2000.18500","volume":"31","author":"K Matsui","year":"2000","unstructured":"Matsui\u00a0K, Tatsuguchi\u00a0A, Valencia\u00a0J et\u00a0al (2000) Extrapulmonary lymphangioleiomyomatosis (LAM): clinicopathologic features in 22\u00a0cases. Hum Pathol 31(10):1242\u20131248. https:\/\/doi.org\/10.1053\/hupa.2000.18500","journal-title":"Hum Pathol"},{"issue":"11","key":"1414_CR14","doi-asserted-by":"publisher","first-page":"1313","DOI":"10.1016\/S2213-2600(21)00228-9","volume":"9","author":"C McCarthy","year":"2021","unstructured":"McCarthy\u00a0C, Gupta\u00a0N, Johnson\u00a0SR, Yu\u00a0JJ, McCormack\u00a0FX (2021) Lymphangioleiomyomatosis: pathogenesis, clinical features, diagnosis, and management. Lancet Respir Med 9(11):1313\u20131327. https:\/\/doi.org\/10.1016\/S2213-2600(21)00228-9","journal-title":"Lancet Respir Med"},{"issue":"5","key":"1414_CR15","doi-asserted-by":"publisher","first-page":"815","DOI":"10.12892\/ejgo3709.2017","volume":"38","author":"H Morita","year":"2017","unstructured":"Morita\u00a0H, Miyahara\u00a0Y, Ueno\u00a0Y, Otani\u00a0K, Kawakami\u00a0F, Ebina\u00a0Y, Yamada\u00a0S (2017) Lymphangioleiomyomatosis observed in the dissected lymph node and myometrium in a\u00a0patient that underwent radical operation for endometrial cancer. EJGO 38(5):815\u2013817. https:\/\/doi.org\/10.12892\/ejgo3709.2017","journal-title":"EJGO"},{"key":"1414_CR16","volume-title":"Afinitor","author":"Novartis Pharma GmbH","year":"2022","unstructured":"Novartis Pharma GmbH (2022) Afinitor (Zulassungsnummer: EU\/1\/09\/538\/009)"},{"key":"1414_CR17","volume-title":"Fachinformation Rapamune \u00ae 0,5\u202fmg\/ 1\u202fmg\/ 2\u202fmg \u00fcberzogene Tabletten","author":"Pfizer Europe","year":"2019","unstructured":"Pfizer Europe (2019) Fachinformation Rapamune \u00ae 0,5\u202fmg\/ 1\u202fmg\/ 2\u202fmg \u00fcberzogene Tabletten (Zulassungsnummer: EU\/1\/01\/171\/013\u2013014)"},{"key":"1414_CR18","doi-asserted-by":"publisher","first-page":"100812","DOI":"10.1016\/j.gore.2021.100812","volume":"37","author":"T Pongsuvareeyakul","year":"2021","unstructured":"Pongsuvareeyakul\u00a0T, Maleki\u00a0S, DeNardo\u00a0BD, Dizon\u00a0DS, Phornphutkul\u00a0C, Singh\u00a0K (2021) Sporadic uterine lymphangioleiomyomatosis (LAM): report of a\u00a0unique case arising in the lower uterine segment with short review. Gynecol Oncol Rep 37:100812. https:\/\/doi.org\/10.1016\/j.gore.2021.100812","journal-title":"Gynecol Oncol Rep"},{"issue":"8","key":"1414_CR19","doi-asserted-by":"publisher","first-page":"1015","DOI":"10.1097\/PAS.0000000000000416","volume":"39","author":"JT Rabban","year":"2015","unstructured":"Rabban\u00a0JT, Firetag\u00a0B, Sangoi\u00a0AR, Post\u00a0MD, Zaloudek\u00a0CJ (2015) Incidental pelvic and para-aortic lymph node lymphangioleiomyomatosis detected during surgical staging of pelvic cancer in women without symptomatic pulmonary lymphangioleiomyomatosis or tuberous sclerosis complex. Am J Surg Pathol 39(8):1015\u20131025. https:\/\/doi.org\/10.1097\/PAS.0000000000000416","journal-title":"Am J Surg Pathol"},{"issue":"10","key":"1414_CR20","doi-asserted-by":"publisher","first-page":"1404","DOI":"10.1097\/PAS.0000000000000470","volume":"39","author":"JK Schoolmeester","year":"2015","unstructured":"Schoolmeester\u00a0JK, Park\u00a0KJ (2015) Incidental nodal lymphangioleiomyomatosis is not a\u00a0harbinger of pulmonary lymphangioleiomyomatosis: a\u00a0study of 19 cases with evaluation of diagnostic Immunohistochemistry. Am J Surg Pathol 39(10):1404\u20131410. https:\/\/doi.org\/10.1097\/PAS.0000000000000470","journal-title":"Am J Surg Pathol"},{"key":"1414_CR21","doi-asserted-by":"publisher","first-page":"12","DOI":"10.1016\/j.humpath.2023.05.006","volume":"138","author":"MC Smithgall","year":"2023","unstructured":"Smithgall\u00a0MC, Liu-Jarin\u00a0X, Chen\u00a0X, Singh\u00a0K, Quddus\u00a0MR, Cimic\u00a0A (2023) Diagnostic utility of PRAME immunohistochemistry in PEComa family of tumors and morphologic mimics with emphasis on the gynecologic tract. Hum Pathol 138:12\u201317. https:\/\/doi.org\/10.1016\/j.humpath.2023.05.006","journal-title":"Hum Pathol"},{"key":"1414_CR22","doi-asserted-by":"publisher","first-page":"390","DOI":"10.1186\/s12885-016-2413-z","volume":"16","author":"K Suzuki","year":"2016","unstructured":"Suzuki\u00a0K, Nagasaka\u00a0K, Oda\u00a0K et\u00a0al (2016) A case of lymphangioleiomyomatosis associated with endometrial cancer and severe systemic lupus erythematosus. BMC Cancer 16:390. https:\/\/doi.org\/10.1186\/s12885-016-2413-z","journal-title":"BMC Cancer"},{"issue":"1","key":"1414_CR23","doi-asserted-by":"publisher","first-page":"55","DOI":"10.1186\/s12931-020-1316-3","volume":"21","author":"Q Wang","year":"2020","unstructured":"Wang\u00a0Q, Luo\u00a0M, Xiang\u00a0B, Chen\u00a0S, Ji\u00a0Y (2020) The efficacy and safety of pharmacological treatments for lymphangioleiomyomatosis. Respir Res 21(1):55. https:\/\/doi.org\/10.1186\/s12931-020-1316-3","journal-title":"Respir Res"},{"key":"1414_CR24","volume-title":"WHO classification of female genital tumours","author":"World Health Organization","year":"2020","unstructured":"World Health Organization (2020) WHO classification of female genital tumours, 5.\u00a0Aufl. Bd. 4. International Agency for Research on Cancer, Lyon","edition":"5"},{"key":"1414_CR25","volume-title":"WHO Classification of tumours thoracic tumours","author":"World Health Organization","year":"2021","unstructured":"World Health Organization (2021) WHO Classification of tumours thoracic tumours, 5.\u00a0Aufl. International Agency for Research on Cancer, Lyon","edition":"5"},{"key":"1414_CR26","doi-asserted-by":"publisher","DOI":"10.1136\/bcr-2021-244257","author":"A Xholli","year":"2021","unstructured":"Xholli\u00a0A, Kratochwila\u00a0C, Vellone\u00a0VG, Schiaffino\u00a0MG (2021) Acute and repeated haemoperitoneum: a\u00a0challenging case of lymphangioleiomyomatosis with uterine PEComa. BMJ Case Rep. https:\/\/doi.org\/10.1136\/bcr-2021-244257","journal-title":"BMJ Case Rep"},{"key":"1414_CR27","doi-asserted-by":"publisher","first-page":"917628","DOI":"10.3389\/fmed.2022.917628","volume":"9","author":"S Xiao","year":"2022","unstructured":"Xiao\u00a0S, Chen\u00a0Y, Tang\u00a0Q, Xu\u00a0L, Zhao\u00a0L, Wang\u00a0Z, Yu\u00a0E (2022) Pelvic lymph node lymphangiomyomatosis found during surgery for gynecological fallopian tube cancer: a\u00a0case report and literature review. Front Med 9:917628. https:\/\/doi.org\/10.3389\/fmed.2022.917628","journal-title":"Front Med"}],"container-title":["Die Pathologie"],"original-title":[],"language":"de","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00292-025-01414-0.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s00292-025-01414-0\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00292-025-01414-0.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,4,29]],"date-time":"2025-04-29T08:58:06Z","timestamp":1745917086000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s00292-025-01414-0"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,2,7]]},"references-count":27,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2025,5]]}},"alternative-id":["1414"],"URL":"https:\/\/doi.org\/10.1007\/s00292-025-01414-0","relation":{},"ISSN":["2731-7188","2731-7196"],"issn-type":[{"type":"print","value":"2731-7188"},{"type":"electronic","value":"2731-7196"}],"subject":[],"published":{"date-parts":[[2025,2,7]]},"assertion":[{"value":"27 December 2024","order":1,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"7 February 2025","order":2,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Einhaltung ethischer Richtlinien"}},{"value":"J.\u00a0M\u00fcller, B.\u00a0Gilks, J.\u00a0McAlpine, G.G.R.\u00a0Hiller, A.K.\u00a0H\u00f6hn und L.-C.\u00a0Horn geben an, dass kein Interessenkonflikt besteht.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Interessenkonflikt"}},{"value":"F\u00fcr diesen Beitrag wurden von den Autor\/-innen keine Studien an Menschen oder Tieren durchgef\u00fchrt. F\u00fcr die aufgef\u00fchrten Studien gelten die jeweils dort angegebenen ethischen Richtlinien.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Interessenkonflikt"}}]}}